Abstract：
:Twenty-four consecutive patients with familial amyloidosis with polyneuropathy (type 1), who were at different stages of the disease were investigated. The purpose was to report the electrophysiological features and to compare them with those found in other generalized neuropathies; 12 cases were familiar and 12 cases were sporadic. The diagnosis was confirmed by examining the occurrence of amyloid substance in rectal or skin biopsies or both. Single fiber EMG with fiber density determination showed signs of collateral innervation, prominent in advanced cases, and a disturbance of neuromuscular function similar to that of progressive spinal motorneuron disease. Action potentials from afferent fibers were not obtained in 91% of the nerves in the lower and 49% of the nerves in the upper extremities. When sensory or motor or slightly subnormal. The neurophysiological findings indicate a symmetrical axonal degeneration, starting in the legs. Familial amyloidosis with polyneuropathy should be suspected in cases of rapidly progressing polyneuropathy of axonal type with onset in middle age, irrespective of whether they are hereditary or not.
journal_name
Acta Neurol Scandjournal_title
Acta neurologica Scandinavicaauthors
Blom S,Steen L,Zetterlund Bdoi
10.1111/j.1600-0404.1981.tb00753.xsubject
Has Abstractpub_date
1981-02-01 00:00:00pages
99-110issue
2eissn
0001-6314issn
1600-0404journal_volume
63pub_type
杂志文章abstract：:Vigabatrin (VGB), an irreversible inhibitor of gamma-aminobutyric acid (GABA) transaminase, is approved as adjunct treatment of refractory partial seizures as well as infantile spasms. Although VGB has been proven to be effective, its use is limited by the risk of retinopathy and associated peripheral visual field def...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章,评审
doi：10.1111/j.1600-0404.2012.01684.x
更新日期：2012-10-01 00:00:00
abstract：OBJECTIVES:To study macrosomatognosia and microsomatognosia occurring as migraine aura symptoms. MATERIAL AND METHODS:Topological distribution of these body schema disturbances were assessed in a collection of 562 pictures submitted as entries to the 4 national Migraine Art competitions. RESULTS:The said symptoms wer...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1034/j.1600-0404.2000.9s334.x
更新日期：2000-06-01 00:00:00
abstract：:We have quantified the pattern of electrical muscle activity at a force of 0.3 kg, 30% of maximum force, and maximum force in the abductor pollicis brevis muscle of 21 patients with neurogenic disorders (10 with carpal tunnel syndrome, 9 with polyneuropathy and 2 with motor neurone disease) and of 15 normal subjects. ...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1984.tb00816.x
更新日期：1984-09-01 00:00:00
abstract：BACKGROUND:The role of electromyography (EMG) recorded from the external anal sphincter (EAS) in the diagnosis of atypical parkinsonian syndromes is a matter for continuous debate. Most studies addressing this issue are retrospective. METHODS:In this study, we prospectively investigated six patients with Parkinson's D...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2009.01169.x
更新日期：2010-03-01 00:00:00
abstract：OBJECTIVES:We sought to assess the efficacy of subthalamic nucleus deep brain stimulation (STN-DBS) in Parkinson's disease (PD)-associated trunk posture abnormalities retrospectively analyzing data from 101 patients reporting mild-to-severe trunk posture abnormalities of a cohort of 216 PD patients treated with STN-DBS...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/ane.12889
更新日期：2018-05-01 00:00:00
abstract：:Serum levels of the soluble interleukin-2 receptor (sIL-2R), an indicator of T cell activation, were significantly elevated in chronic progressive MS (CPMS) patients, clinically stable MS patients and in patients with other neurological diseases (OND) as compared to healthy controls. Levels of sIL-2R in steroid treate...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1991.tb04955.x
更新日期：1991-10-01 00:00:00
abstract：:Botulinum toxin serotype A (BoNT/A) was originally used in neurology for the treatment of dystonia and blepharospasms, but is now clinically used worldwide for the treatment of chronic migraine. Still, the possible mode of action of BoNT/A in migraine is not fully known. However, the mode of action of BoNT/A has been ...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章,评审
doi：10.1111/ane.12906
更新日期：2018-05-01 00:00:00
abstract：BACKGROUND:Far from being uniform, Huntington's disease (HD)'s phenotype encompasses a large variety of motor and non-motor symptoms which occur in various combinations in individual patients. AIM:To describe an unusual association between restless legs syndrome (RLS) and HD. METHODS AND RESULTS:We report a patient w...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2008.01091.x
更新日期：2009-04-01 00:00:00
abstract：:Two cases of increased intracranial pressure associated with lumbar intraspinal tumours are reported. On the basis of protein investigations and constant flow infusion manometric tests, the pathophysiology of the increased intracranial pressure is discussed. It is demonstrated that protein macromolecules leak from the...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：
更新日期：1977-09-01 00:00:00
abstract：OBJECTIVES:To describe clinical, electrophysiological and genetic data of five unrelated Sicilian pedigrees harbouring a heterozygous Ser78Leu mutation in the myelin protein zero (MPZ) extracellular domain. MATERIALS AND METHODS:Clinical, electrophysiological and genetic findings of 16 patients were reported. Polymorp...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2008.01021.x
更新日期：2008-11-01 00:00:00
abstract：:EEG data obtained from 27 patients with presenile Alzheimer's disease (AD) and 28 patients with senile dementia of the Alzheimer type (SDAT) were compared with data from 30 age- and sex-matched controls. Both patient groups exhibited more pronounced delta and theta activity and less prominent alpha and beta activity t...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：
更新日期：1994-01-01 00:00:00
abstract：OBJECTIVES:Fatigue and apathy are two of the most common and most disabling non-motor symptoms of Parkinson's disease (PD). They have a high coincidence and can often be confused; moreover, their relationship is not fully understood. The aim of our study was to describe the coincidence of apathy with different fatigue ...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/ane.12282
更新日期：2015-02-01 00:00:00
abstract：OBJECTIVE:The present study focused on human leukocyte antigen (HLA) DQB1, DQA1 and DRB1 allelic variation according to ethnicity and analyzed whether susceptibility to multiple sclerosis (MS) depends on population characteristics. METHODS:Eighty-eight healthy African-Brazilians and 92 healthy white Brazilians living ...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2006.00750.x
更新日期：2007-05-01 00:00:00
abstract：BACKGROUND:Elevated levels of the cerebrospinal fluid (CSF) neuronal injury markers (neurofilament light chain [NF-L] and total tau protein [t-tau]) and of the astroglial marker glial fibrillary acidic protein (GFAP) are found in etiologically different neurological disorders affecting the peripheral and the central ne...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/ane.13118
更新日期：2019-09-01 00:00:00
abstract：:Myotonic dystrophy seems to have a hypometabolism of non-thyroid origin. The hypometabolism could hypothetically be caused by : 1) reduction in muscle mass, 2) hydrocephalus ex vacuo, an integral part of the disorder, or 3) the basic disease process itself. Possibility 2) was explored by determining basal metabolic ra...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1976.tb04344.x
更新日期：1976-03-01 00:00:00
abstract：OBJECTIVES:Mutations in the CHCHD10 gene, which encodes a mitochondrially targeted protein, have emerged as an important cause of motor neuron disease and frontotemporal lobar degeneration. The aim of this study was to assess the clinical variability in a large family carrying the p.Gly66Val mutation of the CHCHD10 gen...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/ane.12470
更新日期：2016-05-01 00:00:00
abstract：:Muscle cramps are involuntary, painful, sudden contractions of the skeletal muscles. They are present in normal subjects under certain conditions (during a strong voluntary contraction, sleep, sports, pregnancy) and in several pathologies such as myopathies, neuropathies, motoneuron diseases, metabolic disorders, hydr...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章,评审
doi：10.1034/j.1600-0404.2003.01289.x
更新日期：2003-03-01 00:00:00
abstract：:We present clinical and virological data on 9 patients, 7 women and 2 men aged 31-56 years, with recurrent aseptic meningitis (Mollaret's meningitis). Polymerase chain reaction detected Herpes simplex virus type 2 DNA in cerebrospinal fluid samples from all patients collected during their latest attacks of meningitis....
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1998.tb07296.x
更新日期：1998-09-01 00:00:00
abstract：OBJECTIVES:Strokes represent the most common etiology of epilepsy in patients over the age of 60 years, with an incidence of 2-4% occurring in different studies. MATERIALS AND METHODS:In this observational study, 1,428 patients were included who had stroke and were admitted to our Stroke Unit between the years 1996 an...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2006.00642.x
更新日期：2006-07-01 00:00:00
abstract：:The immediate effects of selective sympathetic and somatic blockades on pain and tactile allodynia in 12 patients with long-standing ophthalmic or high cervical postherpetic neuralgia were compared. For the duration of the somatic blockade, pain was completely abolished in 11 patients and allodynia in 8 patients. In c...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1991.tb04923.x
更新日期：1991-08-01 00:00:00
abstract：:We compared orosomucoid levels in cerebrospinal fluid (CSF) in patients with aseptic meningitis, multiple sclerosis, ischemic and hemorrhagic stroke, CNS-affecting leukemia/lymphoma, and CNS-tumor with the levels in a reference group not having neurologic diseases. Because of possible blood brain barrier damage, we co...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1987.tb05454.x
更新日期：1987-05-01 00:00:00
abstract：BACKGROUND:No systematic study has been made to separate age-related clinical deterioration and dysfunctional brain areas from those associated with Parkinson's disease (PD). METHODS:This study included 73 de novo patients with PD and 43 age-matched controls. All subjects underwent [(18)F]-fluorodeoxy glucose (FDG) po...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2010.01446.x
更新日期：2011-09-01 00:00:00
abstract：:The association of cortical tremor, myoclonus and epileptic seizures has been reported in many Japanese and European families with different acronyms. We reviewed the familial cases presenting the clinical picture of autosomal dominant cortical tremor, myoclonus and epilepsy and analysed the phenotypic differences bet...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2005.00385.x
更新日期：2005-04-01 00:00:00
abstract：OBJECTIVES:Focal brain ischemia induced in rodents by occlusion of the middle cerebral artery (MCA) is a widely used paradigm of human brain infarct. The objective of this study is to compare the effectiveness and reproducibility of MCA filament occlusion model in rats and mice. MATERIALS AND METHODS:A total of 140 ro...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1034/j.1600-0404.2003.00010.x
更新日期：2003-04-01 00:00:00
abstract：OBJECTIVE:Ultrasonographic assessment of carotid artery plaque morphology is widely used to identify patients at high risk for stroke. However, the reliability of plaque analysis in high-grade stenosis is uncertain. We determined the interrater reliability of sonographic plaque morphology analysis in patients with seve...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1999.tb00659.x
更新日期：1999-01-01 00:00:00
abstract：:A total of 20 subjects with asymptomatic carotid artery stenosis (ACAS) and a group of healthy controls were compared as to their cognitive performance. Subjects with ACAS showed substantial deficits on tasks of mental speed, learning, visuospatial abilities, verbal processing and deductive reasoning. The cognitive pr...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1991.tb03967.x
更新日期：1991-06-01 00:00:00
abstract：:Patients with prior poliomyelitis with paresis and excessive use (n = 8) and low use (n = 6) of residual anterior tibial motor units (MUs) during walking were subjected to muscle biopsy of the anterior tibial muscle (TA). Antibodies directed against cytoskeletal proteins, spectrin and desmin, and against Leu-19, a myo...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1993.tb04091.x
更新日期：1993-02-01 00:00:00
abstract：:The frequency of post-lumbar puncture headache (PLPH) was registered prospectively in 395 consecutive demented patients at a dementia diagnostic unit. The incidence of PLPH was low, occurring in only 8 patients (2.0%), the severity was mild, and the duration was less than 2 days in all cases but one. The reasons for t...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1993.tb04221.x
更新日期：1993-09-01 00:00:00
abstract：OBJECTIVES:To re-evaluate middle-aged Swedish patients diagnosed with dysequilibrium syndrome (DES) in childhood and to compare their clinical and neuroimaging features to DES with VLDLR gene mutations (DES-VLDR). MATERIALS AND METHODS:Six patients from five families underwent neurological examination and magnetic res...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2010.01335.x
更新日期：2011-01-01 00:00:00
abstract：:Marstock thermotesting evaluates A-delta- and C-fiber functions. To optimize this method, intraindividual variations of vasodilatation, blood flow and sympathetic activity probably biasing thermotest results were imitated by exogenous stimuli which strongly exaggerated these intraindividual variations. In 20 healthy s...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1992.tb08048.x
更新日期：1992-07-01 00:00:00
